Objective. To analyze clinical and serological characteristics of subjects with seleroderma renal crisis (SRC) in Italian patients with systemic sclerosis (SSc). Methods. A retrospective analysis of medical records from 9 Italian rheumatologic referral centers was carried Out. All patients with SRC and an available serum sample at the time of crisis were included. Antinuclear antibodies (ANA) by indirect immunofluorescence, anti-topoisomerase (topo) I by enzyme-linked assay (ELISA), anti-RNA polymerases (RNAP) by ELISA for the subunit 111, and immunoprecipitation (IP) were performed. Results. Forty-six cases (38 female-, 40 diffuse cutaneous SSc) were identified. Mean age at SSc and SRC onset was 52.8 years +/- 13.2 and 55.4 years 11.8, respectively. ANA were present in 44 patients (96%). Anti-topo I antibodies were detected in 30 (65%). anti-RNAP I-III in 7 (15%). No differences emerged between these 2 groups for their main clinical characteristics. The proportion of patients in the anti-RNAP I-III group developing SRC early (< 18 mo) in the course of SSc was significantly higher (p = 0.03). Cumulative survival rates were 64%, 53%, and 35% at 1, 2, and 10 years of followup, respectively. Survival rates of SSc patients significantly differed according to their autoantibody profile, being, lower in the anti-topo I than in the anti-RNAP I-III group (p = 0.034). Conclusion. SRC is a rare manifestation of SSc in Italy but it is still associated with severe prognosis. Anti-topo I reactivity was more frequent than anti-RNAP I-III ill our patients With SRC and was associated with delayed onset and high mortality rates. (First Release June 1 2009; J Rheumatol 2009:36:1464-9: doi: 10.3899/jrheum.080806)
Serologic Profile and Mortality Rates of Scleroderma Renal Crisis in Italy
CODULLO, VERONICA;CAVAGNA, LORENZO;MONTECUCCO, CARLOMAURIZIO
2009-01-01
Abstract
Objective. To analyze clinical and serological characteristics of subjects with seleroderma renal crisis (SRC) in Italian patients with systemic sclerosis (SSc). Methods. A retrospective analysis of medical records from 9 Italian rheumatologic referral centers was carried Out. All patients with SRC and an available serum sample at the time of crisis were included. Antinuclear antibodies (ANA) by indirect immunofluorescence, anti-topoisomerase (topo) I by enzyme-linked assay (ELISA), anti-RNA polymerases (RNAP) by ELISA for the subunit 111, and immunoprecipitation (IP) were performed. Results. Forty-six cases (38 female-, 40 diffuse cutaneous SSc) were identified. Mean age at SSc and SRC onset was 52.8 years +/- 13.2 and 55.4 years 11.8, respectively. ANA were present in 44 patients (96%). Anti-topo I antibodies were detected in 30 (65%). anti-RNAP I-III in 7 (15%). No differences emerged between these 2 groups for their main clinical characteristics. The proportion of patients in the anti-RNAP I-III group developing SRC early (< 18 mo) in the course of SSc was significantly higher (p = 0.03). Cumulative survival rates were 64%, 53%, and 35% at 1, 2, and 10 years of followup, respectively. Survival rates of SSc patients significantly differed according to their autoantibody profile, being, lower in the anti-topo I than in the anti-RNAP I-III group (p = 0.034). Conclusion. SRC is a rare manifestation of SSc in Italy but it is still associated with severe prognosis. Anti-topo I reactivity was more frequent than anti-RNAP I-III ill our patients With SRC and was associated with delayed onset and high mortality rates. (First Release June 1 2009; J Rheumatol 2009:36:1464-9: doi: 10.3899/jrheum.080806)I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.
